Ask the Expert: Dr. Doruk Erkan, Rheumatologist, Answers Your Questions on Antiphospholipid Syndrome
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder in which the immune system makes antibodies (antiphospholipid antibodies or aPL) that increase the risk of forming blood clots and/or miscarriages. Antiphospholipid antibodies can occur in otherwise healthy individuals or in patients with other autoimmune disorders such as systemic lupus erythematosus or rheumatoid arthritis. Primary APS describes aPL positive patients with clots or miscarriages and who do not have evidence of any other autoimmune disorder. About half of APS patients have primary APS.
Q2. What are some indicators that you should reach out to a rheumatologist to find out if you suffer from APS?
There is a wide range of clinical problems related to aPL; deep vein thrombosis is the most common type of blood clot that forms in veins, and stroke is the most common type of blood clot that forms in arteries of APS patients. Another common manifestation of APS is “pregnancy morbidity”, meaning patients may experience certain clinical problems during pregnancy including recurrent miscarriages. In addition to blood clots in relatively young patients and recurrent pregnancy losses, some of the other indicators to reach out to a rheumatologist include low platelet counts, certain types of anemia, heart valve disease, skin rash called “livedo reticularis” (lacey and mottled skin that gives the appearance of blood vessels running beneath the skin in a bluish or reddish color), and false positive syphilis tests. Lastly, aPL may cause abnormal activated partial thromboplastin time (aPTT), which is a clotting test used to determine the bleeding risk before surgical procedures.
Q3. Are there different types of APS? What is the typical treatment of APS?
Having a positive aPL test does not mean the person has APS. The most common medical issues that can occur due to aPL are blood clots and pregnancy-related clinical problems; if these problems develop in aPL-positive individuals, then the APS diagnosis is confirmed. Low platelet counts, heart valve disease, livedo reticularis, aPL-related kidney disease can occur in aPL-positive patients without blood clots or miscarriages. The management of aPL-positive patients depends on the individual patient, his or her aPL-related clinical manifestations, and additional medical conditions. For all patients with positive aPL, whether or not they have had a clotting event, the first essential step is understanding the risk of blood clotting and eliminating other reversible risk factors known to increase the risk of clotting. The main treatments for APS are medications which thin the blood and therefore make clotting less likely. This can be aspirin, anticoagulants (such as warfarin and heparin), or a combination of both, depending on the patient. These medications are routinely used in aPL positive patients with a history of a blood clot or in aPL-positive pregnant women with a history of miscarriages.
Q4. Is there anything that patients can do outside of medicine to help? Diet changes, etc?
Yes, continuous monitoring of cardiovascular disease and blood clot risk factors as well as patient education are crucial in aPL-positive patients to prevent first or recurrent blood clots. Please refer to the Hospital for Special Surgery website articles for further information:
- Blood Clots & Antiphospholipid Antibody (aPL) Positive Patients – Top 10 Points to Assess & Minimize Your Risk
- Lupus & Cardiovascular Disease – Top 10 Points to Assess & Minimize Your Risk
- Cardiovascular Disease Prevention in Lupus Patients
Q5. I was wondering if you believe in the Waxing and Waning of the antibodies? I was diagnosed with Antiphospholipid Antibodies a little over a year ago. I have not had any clots, strokes, or heart attacks. I have had a possible infarction in my left kidney, ITP, Idiopathic Intracranial Hypertension, 2 miscarriages, and 4 preterm labors in the past 14 years. But I am told I can’t be diagnosed with APS, but the pain in my body especially in my legs, headaches, joints, loss of some short term memory, brain fog and severe fatigue really have taken over my life. But recently after two rounds of methotrexate I tested antibodies free. With all these symptoms can the antibodies come and go.
The most commonly used tests to detect antiphospholipid antibodies (aPL) are Lupus Anticoagulant test, Anticardiolipin Antibody Test, and Anti-Beta-2-Gylycoprotein-I Test. Please keep in mind that not every positive laboratory test for aPL is clinically significant. You should discuss with your doctor if your aPL profile is clinically significant (e.g., persistent versus transient aPL, lupus anticoagulant test positive versus negative, triple aPL-positivity versus single aPL positivity, anticardiolipin or anti-Beta-2-Glycoprotein-I tests moderate-to-high positive versus low titer positive). “Waxing and Waning of antibodies” is more common in patients with low-titer anticardiolipin or anti-Beta-2-Glycoprotein-I antibodies.
Q6. I have had leukocytoclastic vasculitis (affecting skin and kidneys) and am on Dapsone which keeps it in remission. Theorized that I have a lupus like condition. Joints ache nearly daily, elevated CRP, etc. I have tested positive for APS for about 8 years but have not thankfully had any clotting issues, carried 2 kids to term and only investigated the clotting issue due to heavy menses. My question is when do we (and how) do we treat it at this point? My rheumatologist is hesitant to treat it at all as I’ve not had any clotting and my titers are on the lower end though they are persistently there. I have been getting minor splinter hemorrhages in thumb nail beds the past few months however and am concerned. I don’t want to rush to unneeded treatment, but don’t want to be reactive AFTER I have my first embolism/stroke. I also have secondary adrenal insufficiency due to Megace therapy for heavy menses if that helps the situation at all. When does one treat the condition or just keep watchful waiting? My rheumatologist is even opposed to daily aspirin at this point.
Please refer to the 5th question to better understand your aPL risk profile and to the 4th question for blood clot prevention strategies in aPL-positive patients. There are no prospective randomized controlled studies demonstrating that daily low-dose aspirin is effective to prevent the first clot in persistently and clinically significant aPL-positive patients; therefore, it is not routinely recommended for aPL-positive patients without history of blood clots. A patient with aPL and other cardiovascular risk factors such as hypertension, diabetes, and/or smoking may be instructed by his or her physician to take a daily low-dose aspirin. I recommend that the general population aspirin guidelines (e.g., by American Heart Association) should be followed to decide about the low dose aspirin treatment in persistently aPL-positive patients without blood clots. As discussed above, the elimination of cardiovascular disease and blood clot risk factors is the most critical step in preventing blood clots in aPL-positive patients. In summary, rather than focusing only on aPL tests, the ideal strategy should be a risk-stratified approach, looking at an individual patient’s medical history and risk factors to devise a blood clot prevention plan.